Pulmonary Fat embolism sickle cell trait

Rationale Acute chest syndrome (ACS) is a life-threatening complication of sickle cell disease (SCD). Current treatment is supportive-supplemental oxygen, transfusions, and antibiotics. Prevention of ACS may reduce morbidity and mortality in patients with SCD. Acute chest syndrome appears similar to pulmonary fat embolism (PFE), a complication of severe skeletal trauma or orthopedic procedures from pulmonary micro-vessel blockage by bone marrow fat. Vascular obstruction and bone marrow necrosis occur in PFE and ACS...

No abstract text is available yet for this article.

S-Homozygotes, SC heterozygotes, and S-beta-thalassaemia heterozygotes are the haemoglobinopathies which make up sickle cell disease. Although their clinical features are similar, as regards complications during pregnancy, Hb S-beta-thalassaemia most dangerous, the main causes of mortality being severe anemia, acute sequestration crisis, bacterial infections, painful episodes, and pulmonary bone marrow fat embolism. Folic acid and antimalarials (where these are indicated) are often successful in preventing severe anaemia...

When a patient with sickle cell disease has fever and a lung infiltrate, usually it will be due to infection, even though cultures may be negative. However, pulmonary infarction can be virtually indistinguishable from pneumonia. Pneumonia is likely to be present in those younger than five years, with purulent sputum and upper lobe infiltrates. Coexisting crisis, a normal or low leukocyte alkaline phosphatase score and microangiopathic changes on peripheral blood smear favor thromboembolic disease. The fat embolism syndrome, caused by bone marrow necrosis and infarction, occurs in sickle cell disease...